The Clinical Significance of Increased Serum Proinflammatory Cytokines, C-Reactive Protein, and Erythrocyte Sedimentation Rate in Patients with Hidradenitis Suppurativa.

OBJECTIVES: To assess inflammatory serum markers including serum proinflammatory cytokines, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) according to the clinical inflammatory activity of patients with hidradenitis suppurativa (HS). PATIENTS AND METHODS: Seventy-four patients with HS were studied based on the Hidradenitis Suppurativa-Physician Global Assessment (HS-PGA) score and Hurley staging system. Proinflammatory cytokines were measured using a multiplex cytokine assay. Twenty-two healthy volunteers were recruited. RESULTS: Serum interleukin- (IL-) 6, IL-23, soluble tumour necrosis factor alpha (TNF-α) receptor I (sTNF-RI), CRP, and ESR were different in the patients with HS compared with those in the healthy controls (P < 0.05). The levels of IL-1ß, IL-6, IL-8, IL-10, IL-12p70, IL-17A, sTNF-RII, CRP, and ESR were significantly elevated according to inflammatory activity based on HS-PGA scores (r > 0.25, P < 0.05). The levels of IL-6 (r = 0.53, P < 0.001), CRP (r = 0.54, P < 0.001), and ESR (r = 0.60, P < 0.001) were especially well correlated with clinical inflammatory activity based on HS-PGA scores. The levels of IL-6, IL-8, sTNF-RI, sTNF-RII, CRP, and ESR were significantly elevated according to Hurley staging system. CONCLUSIONS: Serum proinflammatory cytokines, CRP, and ESR are increased in relation to the clinical inflammatory activity of patients with HS compared with healthy controls. Serum IL-6, CRP, and ESR are effective biomarkers for evaluating the severity of HS.

Trastornos genéticos con eccema moderado-grave refractario y elevación de inmunoglobulina E: diagnóstico diferencial / Differential Diagnosis of Genetic Disorders Associated with Moderate to Severe Refractory Eczema and Elevated Immunoglobulin E

La asociación de eccema moderado-grave y niveles elevados de IgE en plasma es característica no solo de la dermatitis atópica, sino también de diversas genodermatosis: síndromes hiper-IgE, síndrome de Omenn, síndrome de Netherton, síndrome de la piel exfoliada tipo B, síndrome de dermatitis grave-alergias múltiples-desgaste metabólico, síndrome de Wiskott-Aldrich, déficit de prolidasa, síndrome de Loeys-Dietz, síndrome IPEX, déficit de STAT5B y pentasomía X. Se trata de pacientes pediátricos que presentan un cuadro clínico compatible con dermatitis atópica grave, con mala respuesta a los tratamientos clásicos y que asocian elevación de IgE desde el nacimiento. Además, comparten con frecuencia otras manifestaciones clínicas y analíticas, lo cual dificulta el diagnóstico. Presentamos una guía práctica para orientar el diagnóstico diferencial entre todas estas entidades y, por lo tanto, ayudar a decidir cuándo y el tipo de test genético a realizar para establecer el diagnóstico definitivo

The association of moderate to severe eczema and elevated plasma levels of immunoglobulin E is a characteristic not only of atopic dermatitis but also of various genodermatoses: hyperimmunoglobulin E syndromes, Omenn syndrome, Netherton syndrome, peeling skin syndrome type B, severe dermatitis, multiple allergies, and metabolic wasting syndrome, Wiskott-Aldrich syndrome, prolidase deficiency, Loeys-Dietz syndrome, IPEX syndrome, STAT5B deficiency, and pentasomy X. The clinical presentation of these genodermatoses -typically in children- is consistent with severe atopic dermatitis. Immunoglobulin E is elevated from birth and response to conventional treatments is poor. Diagnosis is further complicated by the fact that these genodermatoses often share other clinical manifestations and laboratory findings. We present practical guidelines for differentiating among these various entities, with the aim of helping physicians decide what type of genetic test should be carried out –and when– in order to establish a definitive diagnosis

Differential Diagnosis of Genetic Disorders Associated with Moderate to Severe Refractory Eczema and Elevated Immunoglobulin E.

The association of moderate to severe eczema and elevated plasma levels of immunoglobulin E is a characteristic not only of atopic dermatitis but also of various genodermatoses: hyperimmunoglobulin E syndromes, Omenn syndrome, Netherton syndrome, peeling skin syndrome type B, severe dermatitis, multiple allergies, and metabolic wasting syndrome, Wiskott-Aldrich syndrome, prolidase deficiency, Loeys-Dietz syndrome, IPEX syndrome, STAT5B deficiency, and pentasomy X. The clinical presentation of these genodermatoses -typically in children- is consistent with severe atopic dermatitis. Immunoglobulin E is elevated from birth and response to conventional treatments is poor. Diagnosis is further complicated by the fact that these genodermatoses often share other clinical manifestations and laboratory findings. We present practical guidelines for differentiating among these various entities, with the aim of helping physicians decide what type of genetic test should be carried out -and when- in order to establish a definitive diagnosis.

Ictiosis arlequín tratada con acitretino / Treatment of Harlequin Ichthyosis With Acitretin

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Melanoma intradérmico asociado a nevo melanocítico intradérmico / Intradermal Melanoma Associated With an Intradermal Melanocytic Nevus

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Vasculopatía oclusiva por crioglobulinemia tipo I en mieloma múltiple IgG lambda / Occlusive vasculopathy due to type I cryoglobulinemia in IgG-lambda type multiple myeloma

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Enfermedad de Dowling-Degos: caso clínico asociado a hidradenitis supurativa / Dowling-Degos Disease Associated With Hidradenitis Suppurativa: A Case Report

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Vasculitis necrosante inducida por cocaína / Necrotizing vasculitis induced by cocaine

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Vendajes húmedos: nuestra experiencia / Our Experience With Wet-Wrap Treatment

En la actualidad disponemos de un importante arsenal terapéutico para la dermatitis atópica grave. Entre los tratamientos sistémicos cabe destacar entre otros la ciclosporina, los glucocorticoides, la azatioprina, el metotrexato, el mofetil micofenolato o el omalizumab. La terapia con vendajes húmedos oclusivos (wet-wrap) puede suponer una excelente alternativa en pacientes en los que se pretende evitar o reducir el uso de tratamientos sistémicos. Hasta el momento los vendajes húmedos se han considerado como una alternativa en los casos de dermatitis atópica grave de la infancia. Aportamos nuestra experiencia en un grupo de 7 pacientes adultos, 5 de ellos con dermatitis atópica y 2 con prurigo nodular, destacando los resultados satisfactorios obtenidos, así como los escasos efectos secundarios observados

A wide range of treatments are currently available for severe atopic dermatitis, including systemic therapies such as ciclosporin, corticosteroids, azathioprine, methotrexate, mofetil mycophenolate, and omalizumab. In patients who can no longer take systemic drugs or who need a dose reduction, wet-wrap treatment can be an excellent option. To date, wet wraps have mostly been used in severe cases of childhood atopic dermatitis. We report our experience with wet-wrap treatment in 5 adults with atopic dermatitis and 2 with nodular prurigo. The results were satisfactory and there were few adverse effects

Our experience with wet-wrap treatment.

A wide range of treatments are currently available for severe atopic dermatitis, including systemic therapies such as ciclosporin, corticosteroids, azathioprine, methotrexate, mofetil mycophenolate, and omalizumab. In patients who can no longer take systemic drugs or who need a dose reduction, wet-wrap treatment can be an excellent option. To date, wet wraps have mostly been used in severe cases of childhood atopic dermatitis. We report our experience with wet-wrap treatment in 5 adults with atopic dermatitis and 2 with nodular prurigo. The results were satisfactory and there were few adverse effects.

Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine.

Cocaine abuse is associated with various skin and rheumatological diseases that mimic primary autoimmune diseases, including retiform purpura with involvement of the ears, cocaine-induced midline destructive lesions (CIMDL), and eruptive pyoderma gangrenosum (PG). Previous reports have suggested the use of perinuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE) to differentiate these cocaine-induced diseases from primary autoimmune diseases. We describe a case of a 54-year-old woman with a history of cocaine abuse, who had PG lesions on her legs with accompanying CIMDL and lung lesions similar to those seen in Wegener granulomatosis. Detection of HNE-positive pANCA, and improvement or clinical recurrence after cessation or consumption of cocaine, respectively, were key to differentiating this presentation from primary autoimmune disease.

Tuberculosis cutánea periorificial vulvar / Periorificial cutaneous tuberculosis of the vulva

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